Longitudinal development of hand function in children with unilateral cerebral palsy

Aim  The aim of this study was to describe how the usefulness of the hemiplegic hand develops in children with unilateral cerebral palsy (CP) aged between 18 months and 8 years. Method  A prospective longitudinal study of 43 children (22 males, 21 females) with unilateral CP was conducted. Inclusion age was 18 months to 5 years 4 months (mean 2y 8mo [SD 1y 1mo]). Children were assessed with the Assisting Hand Assessment (AHA) 3 to 11 times per child over a mean period of 4 years 6 months. Two models were used for grouping children: by AHA score at 18 months and by Manual Ability Classification System (MACS) levels. Estimated average motor development curves were fitted with a nonlinear mixed‐effects model. Results  Children with a high AHA score (high ability level) at 18 months reached a significantly higher ability level and at a higher progression rate than children with a low 18‐month AHA score. Limits of development differed between the three MACS levels. Interpretation  Results indicate that the AHA score at 18 months can be used to discuss future development of affected hand use in bimanual tasks in children with unilateral CP.     

Initial experience with off-pump left ventricular assist device implantation in single center: retrospective analysis

Background  

We hypothesize that implantation of left ventricular assist device through off-pump technique is feasible and has a comparable result to implantation on cardiopulmonary bypass and could improve one-year survival.     

Compound muscle action potential and motor function in children with spinal muscular atrophy

Reliable outcome measures that reflect the underlying disease process and correlate with motor function in children with SMA are needed for clinical trials. Maximum ulnar compound muscle action potential (CMAP) data were collected at two visits over a 4–6‐week period in children with SMA types II and III, 2–17 years of age, at four academic centers. Primary functional outcome measures included the Modified Hammersmith Functional Motor Scale (MHFMS) and MHFMS‐Extend. CMAP negative peak amplitude and area showed excellent discrimination between the ambulatory and non‐ambulatory SMA cohorts (ROC = 0.88). CMAP had excellent test–retest reliability (ICC = 0.96–0.97, n = 64) and moderate to strong correlation with the MHFMS and MHFMS‐Extend (r = 0.61–0.73, n = 68, P < 0.001). Maximum ulnar CMAP amplitude and area is a feasible, valid, and reliable outcome measure for use in pediatric multicenter clinical trials in SMA. CMAP correlates well with motor function and has potential value as a relevant surrogate for disease status. Muscle Nerve, 2010     

Neurodevelopmental outcomes in children with cerebellar malformations: a systematic review

Cerebellar malformations are increasingly diagnosed in the fetal period. Consequently, their consideration requires stressful and often critical decisions from both clinicians and families. This has resulted in an emergent need to understand better the impact of these early life lesions on child development. We performed a comprehensive literature search of studies describing neurodevelopmental outcomes of cerebellar malformations between January 1997 and December 2007. Overall, the data suggested that children with isolated inferior vermis hypoplasia (IVH) and mega cisterna magna (MCM) have a good developmental outcome, whereas children with molar tooth sign/Joubert syndrome, vermis hypoplasia, pontocerebellar hypoplasia (PCH) type II, and cerebellar agenesis experience moderate to severe global developmental delays. Reports for Dandy−Walker malformation (DWM) were conflicting; however, the presence of a normally lobulated vermis and the absence of associated brain anomalies were associated with a more favourable outcome. Finally, children with isolated cerebellar hypoplasia experienced fewer impairments. Important methodological limitations highlighted include a lack of standardized outcome measure use in 79% of studies and the predominant use of retrospective study designs (85%), with 40% limited to case reports or case-series. In summary, rigorous outcome studies describing the spectrum of disabilities in survivors are urgently needed to accurately delineate the long-term neurodevelopmental consequences of cerebellar malformations.